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    • Basic and Clinical Science Course - Excerpt
  • 2020–2021 BCSC Basic and Clinical Science Course™

    Go to Academy Store Learn more and Purchase.

    1 Update on General Medicine

    Chapter 11: Behavioral and Neurologic Disorders

    Neurologic Disorders

    Alzheimer Disease and Dementia

    Dementia is a disorder characterized by a decline in cognitive ability to a point of interfering with daily function. The diagnosis can be challenging due to its insidious onset; early symptoms may only be apparent to close family members. Globally, the prevalence of dementia in individuals aged 60 and over is between 5%–8%. Of the 50 million people affected worldwide, approximately 60% live in low- to middle-income regions. Several specific syndromes fall under the category of dementia, including Alzheimer disease, vascular (multi-infarct) dementia, and Lewy body dementia.

    Alzheimer disease

    Alzheimer disease (AD) is the most common cause of dementia in people older than 65 years. Memory impairment is its cardinal feature, with language and behavioral deficits occurring over time. In addition to age, family history appears to be a risk factor, suggesting a genetic link; the early-onset form of the disease seems to have the strongest genetic tie. The pathologic hallmarks of AD are extraneuronal amyloid plaques and neurofibrillary degeneration. These 2 findings are associated with neuronal death and decreased levels of the neurotransmitter acetylcholine. As the disease progresses, the basal forebrain and eventually the cerebral cortex become involved.

    Diagnosis of AD is made clinically; serological testing and neuroimaging studies are used to rule out other causes. Epidemiological data from the European Community Concerted Action Epidemiology of Dementia Group (EURODEM) found that 70% of patients with dementia have AD. In 2015, the estimated global cost of the disease was $808 billion. Life expectancy in individuals with AD is shortened relative to the degree of impairment at the time of diagnosis. The disease presents significant challenges to family and caregivers in dealing with a variety of related issues, including emotional lability, risk of wandering, and potential for injury. Resources are available to assist patients and their families with these matters, such as the Alzheimer’s Association (www.alz.org).

    An atypical presentation of AD can result from neuropathological abnormalities concentrated in particular areas of the brain. For example, posterior cortical atrophy can lead to progressive cortical impairment and ocular manifestations from pathology that involves the visual pathways. As a result, many of these patients may present early in the progression of their disease to an optometrist or ophthalmologist with a variety of visual symptoms and findings, including homonymous visual field defects.

    Cholinesterase inhibitors such as donepezil and the neuropeptide-modifying agent memantine are helpful, used either alone or in combination therapy, in treating patients with AD. Studies investigating the potential benefit of vitamin E supplementation continue to show mixed results.

    Vascular dementia

    Vascular dementia is the second most common form of dementia and accounts for 10%–20% of cases in North America and Europe. The disease is associated with findings on neurologic examination consistent with prior strokes; neuroimaging studies typically show evidence of multiple infarcts. As in other vascular diseases, patients with hypertension, diabetes mellitus, or abnormal lipid profiles are at increased risk. Although donepezil and memantine are sometimes used in treatment, their benefit appears to be limited. Management is usually directed at treating any comorbidities, including the behavioral symptoms that often accompany this disease.

    Lewy body dementia

    Lewy body dementia (LBD) is another common form of neurodegenerative dementia. The disease is characterized pathologically by the presence of eosinophilic intracytoplasmic inclusions (Lewy bodies) in the deep cortical regions of the brain. There may be considerable clinical and pathologic overlap between LBD, AD, and Parkinson disease. Ophthalmologists should be aware of LBD, however, because patients with this syndrome often present with complex (or formed) visual hallucinations. The Dementia with Lewy Body Consortium has recently revised the criteria used to diagnose the disorder, including interpretation of certain biomarkers and the significance of the presence of an REM sleep behavior disorder in the patient. There are no specific pharmacotherapy options for patients affected, although cholinesterase inhibiters have shown some benefit in select cases.

    Ophthalmic considerations Patients with dementia may report a host of visual symptoms and manifest a variety of findings depending on the extent of the disease. Reduced contrast sensitivity, depth perception, and motion perception have been reported in cases of Alzheimer disease (AD). Because this disease is associated with an impaired cholinergic system, a reduced pupillary constriction response may be observed, which improves after treatment with the anticholinesterase agent donepezil.

    Unfortunately, there is currently no reliable test specific to the diagnosis of AD. Ocular motility disorders, especially saccadic latency, have been observed. Because the retina is an extension of the nervous system, researchers are investigating ways to help detect neurodegenerative disease using optical coherence tomography (OCT); thinning of the retinal nerve fiber layer (RNFL) may be an associated finding.

    • James BD, Leurgans SE, Hebert LE, Scherr PA, Yaffe K, Bennett DA. Contribution of Alzheimer disease to mortality in the United States. Neurology. 2014;82(12):1045–1050.

    • Moretti, D, ed. Update on Dementia. London, United Kingdom: InTechOpen Limited; 2016.

    Excerpted from BCSC 2020-2021 series: Section 1 - Update on General Medicine. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.

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    2022-2023 Basic and Clinical Science Course, Complete Print Set
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    2022-2023 Basic and Clinical Science Course Residency Set
    2022-2023 Basic and Clinical Science Course, Section 01: Update on General Medicine
    2022-2023 Basic and Clinical Science Course, Section 02: Fundamentals and Principles of Ophthalmology
    2022-2023 Basic and Clinical Science Course, Section 03: Clinical Optics and Vision Rehabilitation
    2022-2023 Basic and Clinical Science Course, Section 04: Ophthalmic Pathology and Intraocular Tumors
    2022-2023 Basic and Clinical Science Course, Section 05: Neuro-Ophthalmology
    2022-2023 Basic and Clinical Science Course, Section 06: Pediatric Ophthalmology and Strabismus
    2022-2023 Basic and Clinical Science Course, Section 07: Oculofacial Plastic and Orbital Surgery
    2022-2023 Basic and Clinical Science Course, Section 08: External Disease and Cornea
    2022-2023 Basic and Clinical Science Course, Section 09: Uveitis and Ocular Inflammation
    2022-2023 Basic and Clinical Science Course, Section 10: Glaucoma
    2022-2023 Basic and Clinical Science Course, Section 11: Lens and Cataract
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